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The aim of this study was to determine the characteristics, treatment, and outcome according to each etiology of pachymeningitis.We conducted a retrospective multicenter French nationwide study between 2000 and 2016 to describe the characteristics, outcome, and treatment of pachymeningitis.We included 60 patients (median age 55.5 years; interquartile range [IQR] 30-80, female/male ratio 0.43). Neurologic signs were present in 59 patients (98%) and consisted of headache in 43 (72%), cranial nerve palsy in 33 (55%), confusion in 10 (17%), seizures in 7 (12%), and focal neurologic signs in 9 (15%). Fever and weight loss were present in 8 (13%) and 13 cases (22%), respectively. Cerebral venous thrombosis was present in 8 cases (13%). Analysis of cerebrospinal fluid showed moderate hyperproteinorachia (median 0.68 g/L; IQR 0.46-3.2) with or without pleiocytosis. Diagnosis included idiopathic pachymeningitis (n = 18; 30%); granulomatosis with polyangiitis (n = 13; 17%); Erdheim-Chester disease (n = 10; 17%); IgG4-related disease and tuberculosis (n = 3; 5% each); Rosai-Dofman disease, microscopic polyangiitis, and sarcoidosis (n = 2, 3% each); cryptococcal meningitis, Lyme disease, ear-nose-throat infection, postlumbar puncture, low spinal-fluid pressure syndrome, and lymphoma (n = 1 each). We found no difference in demographics and neurologic presentation among idiopathic pachymeningitis, Erdheim-Chester disease, and granulomatosis with polyangiitis. In contrast, frequencies were lower with idiopathic pachymeningitis than Erdheim-Chester disease for general signs (6% and 40%, respectively, P = .041) and complete neurologic response (0% vs 39%, P = .045).The detection of extraneurologic signs and routine screening are needed to classify the pachymeningitis origin. Prospective studies are warranted to determine the best treatment in each case.

Background: As intravenous immunoglobulins (IVIG) exhibit immunomodulatory and antifibrotic properties, they may be a relevant treatment for systemic sclerosis (SSc). The objectives of this work were thus to report on the efficacy and safety of IVIG in a population of SSc patients and to review the available literature., Methods: 46 patients from 19 French centers were retrospectively recruited. They were included if they had a diagnosis of SSc and received at least 1 IVIG infusion at a dosage >1g/kg/cycle. Relevant data collected at IVIG discontinuation were compared to those collected at IVIG initiation. A comprehensive literature review was performed., Results: We observed a significant improvement of muscle pain (74% vs. 20%, p<0.0001), muscle weakness (45% vs. 21%, p=0.01), joint pain (44% vs. 19%, p=0.02), CK levels (1069±1552UI vs. 288±449UI, p<0.0001) and CRP levels (13.1±17.6mg/L vs. 9.2±16.6mg/L, p=0.001). We also noted a trend for an improvement of gastro-esophageal reflux disease (68% vs. 53%, p=0.06) and bowel symptoms (42% vs. 27%, p=0.06). Skin and cardiorespiratory involvements remained stable. Finally, corticosteroid daily dose was significantly lower by the end of treatment (13.0±11.6mg/day vs. 8.9±10.4mg/day, p=0.01). Only two severe adverse events were reported (one case of deep vein thrombosis and one case of diffuse edematous syndrome)., Conclusion: Our work suggests that IVIG are a safe therapeutic option that may be effective in improving musculoskeletal involvement, systemic inflammation, digestive tract symptoms and could be corticosteroid sparing., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Introduction: Adult-onset Still's disease is characterized by non-specific polymorphic features. The efficacy of anakinra, an IL-1 receptor antagonist, has been shown in several studies. This medication is well-tolerated, and only one case of severe hepatitis has been previously reported., Case Report: A 22-year-old woman presented with fever, rash, arthritis, and pericarditis, associated with systemic inflammatory response syndrome and elevated ferritin serum level with low glycosylated ferritin. Adult-onset Still's disease was diagnosed, but treatment with steroids did not achieve remission. The patient was then treated with anakinra, which resulted in spectacular improvement, but 3 weeks after the initiation of the treatment she experienced severe hepatitis that resolved after the discontinuation of anakinra., Conclusion: Hepatitis is a rare side effect of anakinra and the monitoring of liver tests should be recommended during anakinra therapy., (Copyright © 2012 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

A 56-year-old woman with ankylosing spondylitis, treated for 3 months with infliximab, developed miliary tuberculosis with mediastinal lymphadenopathies and brain and splenic lesions. After initial improvement under anti-tuberculous therapy, she suffered an unexpectedly prolonged paradoxical worsening with several episodes of lymphadenopathy, including life-threatening ones, over a period of more than 14 months of follow-up. The outcome was favorable as a result of corticosteroid and surgical treatments. This phenomenon reflects a paradoxical reaction precipitated by infliximab withdrawal., (Copyright © 2010 International Society for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.)

Introduction: Fever during a myelodysplastic syndrome can be due to infectious complications, systemic disease or acute transformation with clonal evolution., Case Report: A 51-year-old woman, with a 5q- syndrome and neutropenia, presented with a several week fever duration. Infectious work-up was negative and therapy with antibiotics had no influence on the clinical course. Neither bone marrow nor blood blasts were detected, but liver biopsy demonstrated significant blast infiltration compatible with the diagnosis of acute myeloid leukaemia (AML)., Conclusion: The absence of blasts in blood or bone marrow does not exclude the malignant transformation of a myelodysplastic syndrome to AML. Tissue biopsy may be necessary to confirm the leukaemic progression.

We report a case of dengue fever with plasma cells in the blood (3980 per cubic millimeter) and bone marrow (30%) in a 55-year-old woman hospitalized for fever, arthralgias and thrombocytopenia (66,000 per cubic millimeter) on returning from the West Indies. Serological testing confirmed the diagnosis. Plasmacytosis is rare in dengue fever and its frequency and correlation with the different forms of the disease remain to be determined.